INTRATHECAL BACLOFEN THERAPY FOR STIFF-MAN SYNDROME AND PROGRESSIVE ENCEPHALOMYELOPATHY WITH RIGIDITY AND MYOCLONUS

We report on eight patients with stiff-man syndrome (SMS) or its ''plu s'' variant, progressive encephalomyelopathy with rigidity and myoclon us (PERM) receiving intrathecal baclofen via pump. In six of the patie nts, follow-ups continued for approximately 2.5 to 6.5 years after pum p implantation. Intrathecal baclofen was an effective last-resort alte rnative for patients who responded poorly to or did not tolerate oral antispasticity medications. General mobility increased, and spasms and rigidity were reduced; however, no complete remissions were observed either before or after pump implantation. PERM patients showed more se vere and rapid progression of symptoms and more attacks of autonomic d ysregulation than SMS patients. They also required higher doses and mo re rapid dosage increases. Complications of intrathecal baclofen thera py included spasm-induced rupture of the catheter, catheter dislocatio n causing radicular symptoms, and pump malfunction resulting in inaccu rate dosage administration. Patients suffered fewer side effects with intrathecal baclofen than with oral medication, but overdose resulted in a transient, comalike state in one patient and sudden dosage reduct ion due to pump failure was fatal in another.